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Special Issue "Wernicke's Encephalopathy and Korsakoff's Syndrome"

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A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Clinical Neurology".

Deadline for manuscript submissions: 20 September 2023 | Viewed by 5224

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Special Issue Editors

Prof. Dr. Roy P.C. Kessels

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Guest Editor

Donders Institute for Brain, Cognition and Behaviour, Radboud University, Nijmegen, The Netherlands
Interests: cognitive and clinical neuropsychology; neuropsychiatry

Prof. Dr. Cees M.P.M. Hertogh

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Guest Editor

Department of Medicine for Older People, Amsterdam Public Health Research Institute, Amsterdam University Medical Centres, Location VUmc, Amsterdam, The Netherlands
Interests: medical care in advanced disease; geriatric palliative care; care ethics

Dr. Erik Oudman

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Guest Editor

Lelie Care Group, Slingedael Korsakoff Center, Rotterdam, The Netherlands
Interests: nonpharmacological treatment; long-term care; neuropsychology; neuropsychiatry

Special Issue Information

Dear Colleagues,

Wernicke’s encephalopathy and Korsakoff’s syndrome are neuropsychiatric syndromes that may occur in the context of malnutrition and thiamine deficiency, often—but not exclusively—in the context of alcohol use disorder. Despite the fact that thiamine supplementation in individuals at risk for developing Korsakoff’syndrome is available, there are still many patients that are newly diagnosed worldwide, alhough exact numbers are lacking. In addition, many people who have been diagnosed with Korsakoff’s syndrome still have to cope with the long-term consequences of this disorder that affect the everyday life of these individuals. In this Special Issue, we welcome authors to submit papers on Wernicke’s encephalopathy and/or Korsakoff’s syndrome. Topics may include—but are not limited to—their assessment and diagnostic work-up, their neuropsychiatric or cognitive sequelae, the neuropathology of these syndromes, somatic comorbidity, pharmacological and nonpharmacological treatments, quality of life, mental capacity, patient care and nursing, epidemiology, or neuroimaging research. Note that we welcome submissions on both alcoholic and nonalcoholic Wernicke’s encephalopathy and Korsakoff’s syndrome.

Prof. Dr. Roy P.C. Kessels
Prof. Dr. Cees M.P.M. Hertogh
Dr. Erik Oudman
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

alcohol-related cognitive disorders
neuropsychiatry
neuropsychology
thiamine
addiction
nursing

Published Papers (6 papers)

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Research

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Open AccessArticle

Coordination and Cognition in Pure Nutritional Wernicke’s Encephalopathy with Cerebellar Degeneration after COVID-19 Infection: A Unique Case Report

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J. Clin. Med. 2023, 12(7), 2511; https://doi.org/10.3390/jcm12072511 - 27 Mar 2023

Viewed by 549

Abstract

Background: Alcoholic cerebellar degeneration is a restricted form of cerebellar degeneration, clinically leading to an ataxia of stance and gait and occurring in the context of alcohol misuse in combination with malnutrition and thiamine depletion. However, a similar degeneration may also develop after non-alcoholic malnutrition, but evidence for a lasting ataxia of stance and gait and lasting abnormalities in the cerebellum is lacking in the few patients described with purely nutritional cerebellar degeneration (NCD). Methods: We present a case of a 46-year-old woman who developed NCD and Wernicke’s encephalopathy (WE) due to COVID-19 and protracted vomiting, resulting in thiamine depletion. We present her clinical course over the first 6 months after the diagnosis of NCD and WE, with thorough neuropsychological and neurological examinations, standardized clinical observations, laboratory investigations, and repeated MRIs. Results: We found a persistent ataxia of stance and gait and evidence for an irreversible restricted cerebellar degeneration. However, the initial cognitive impairments resolved. Conclusions: Our study shows that NCD without involvement of alcohol neurotoxicity and with a characteristic ataxia of stance and gait exists and may be irreversible. We did not find any evidence for lasting cognitive abnormalities or a cerebellar cognitive-affective syndrome (CCAS) in this patient. Full article

(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)

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Open AccessArticle

Distinct Sleep Alterations in Alcohol Use Disorder Patients with and without Korsakoff’s Syndrome: Relationship with Episodic Memory

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J. Clin. Med. 2023, 12(6), 2440; https://doi.org/10.3390/jcm12062440 - 22 Mar 2023

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Abstract

Alcohol Use Disorder (AUD) results in sleep disturbances that may have deleterious impacts on cognition, especially on memory. However, little is known about the sleep architecture in patients with Korsakoff’s syndrome (KS). This study aims at characterizing sleep disturbances in KS compared to AUD without KS and at specifying the relationships with cognitive impairments. Twenty-nine AUD patients (22 without KS and 7 with KS) and 15 healthy controls underwent a neuropsychological assessment and a polysomnography. The severity of sleep-disordered breathing and sleep fragmentation was similar in AUD and KS patients compared to controls. Sleep architecture differed between both patient groups: the proportion of slow-wave sleep was reduced in AUD patients only, while a lower proportion of rapid-eye movement (REM) sleep was specifically observed in KS patients. The proportion of REM sleep correlated with the severity of episodic memory deficits when AUD and KS were examined together. These data provide evidence for both similarities and specificities regarding sleep alterations in AUD patients with and without KS. They also indicate that altered sleep architecture may contribute to the pathophysiology of alcohol-related memory disorders. Full article

(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)

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Open AccessArticle

Factors Associated with ADL Dependence in Nursing Home Residents with Korsakoff’s Syndrome and Other Alcohol-Related Disorders: An Explorative Cross-Sectional Study

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J. Clin. Med. 2023, 12(6), 2181; https://doi.org/10.3390/jcm12062181 - 11 Mar 2023

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Abstract

Difficulties in performing activities of daily living (ADL) are common in patients with Korsakoff‘s syndrome (KS). The aim of this study was to identify factors associated with ADL dependence in nursing home residents with KS. This exploratory, cross-sectional study included 281 residents with KS from 9 specialized nursing homes in the Netherlands. We examined demographic, cognitive, somatic, and (neuro)psychiatric characteristics. ADL dependence was assessed with the Inter-RAI ADL Hierarchy Scale. Multivariable logistic regression analyses were used to identify factors associated with ADL dependence. Cognitive impairment (odds ratio [OR] = 7.46; 95% confidence interval [CI] = 2.10–30.5), female gender (OR = 3.23; CI, 1.21–8.78), staying in a nursing home for ≥5 years (OR = 3.12; CI, 1.24–8.33), and impaired awareness (OR = 4.25; CI, 1.56–12.32) were significantly associated with higher ADL dependence. Chronic obstructive pulmonary disease (COPD) was significantly associated with lower ADL dependence (OR = 0.31; CI, 0.01–0.84). The model explained 32% of the variance. The results suggest that when choosing interventions aimed at improving ADL functioning, special attention should be paid to residents living more than five years in the nursing home, with a female gender, with more severe cognitive impairments, and/or with COPD. Full article

(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)

Open AccessArticle

“What Did I Tell This Sad Person?”: Memory for Emotional Destinations in Korsakoff’s Syndrome

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J. Clin. Med. 2023, 12(5), 1919; https://doi.org/10.3390/jcm12051919 - 28 Feb 2023

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Abstract

We investigated destination memory, defined as the ability to remember to whom a piece of information was previously transmitted, for emotional destinations (i.e., a happy or sad person) in Korsakoff’s syndrome (KS). We asked patients with KS and control participants to tell facts to neutral, positive, or negative faces. On a subsequent recognition task, participants had to decide to whom they told each fact. Compared with control participants, patients with KS demonstrated lower recognition of neutral, emotionally positive, and emotionally negative destinations. Patients with KS demonstrated lower recognition of emotionally negative than for emotionally positive or neutral destinations, but there were no significant differences between recognition of neutral and emotionally positive destinations. Our study demonstrates a compromised ability to process negative destinations in KS. Our study highlights the relationship between memory decline and impaired emotional processing in KS. Full article

(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)

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Open AccessArticle

The Use of an Errorless Learning Application to Support Re-Learning of (Instrumental) Activities for People Living with Korsakoff Syndrome

Roeline Biemond

Erik Oudman

and

Albert Postma

J. Clin. Med. 2022, 11(23), 6947; https://doi.org/10.3390/jcm11236947 - 25 Nov 2022

Cited by 1 | Viewed by 639

Abstract

Korsakoff syndrome (KS) is a severe neuropsychiatric syndrome derived from acute thiamine deficiency and concomitant alcohol use disorders. KS patients need lifelong assistance because of the severity of their cognitive problems. In clinical practice and research, errorless learning has proven to be an effective cognitive rehabilitation method for patients with KS. Our study focused on optimizing errorless learning by introducing new software technology to support the training process of errorless learning. Although the benefits of errorless learning for patients with Korsakoff’s syndrome have been thoroughly investigated, it is currently unclear whether new technology could contribute to better learning and maintenance of everyday tasks. Therefore, an errorless learning application was built. This device is a web application and can be used on a tablet, laptop, or smartphone. The application allows clinicians and researchers to insert pictures, videoclips, timers, and audio fragments in the different steps of an errorless learning training plan. This way, the different steps are visible and easy to follow for patients. Moreover, it ensures as a learning method that the training is executed exactly the same way for each and every training. The aim of this study was twofold: to examine whether the use of the errorless learning application is effective, and whether it leads to better results than a regular errorless learning of everyday activities. In total, 13 patients with KS were trained in instrumental activities of daily living by means of the application, and 10 patients were trained with traditional instructions. Results showed an equal improvement for both training methods. Importantly, the technology group could better remember the training when probed at a later moment than the traditional errorless learning group. These results are promising for further development of novel technology to support errorless learning applications in clinical practice. Full article

(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)

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Review

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Open AccessReview

A Clinician’s View of Wernicke-Korsakoff Syndrome

Jan W. Wijnia

J. Clin. Med. 2022, 11(22), 6755; https://doi.org/10.3390/jcm11226755 - 15 Nov 2022

Cited by 1 | Viewed by 1901

Abstract

The purpose of this article is to improve recognition and treatment of Wernicke-Korsakoff syndrome. It is well known that Korsakoff syndrome is a chronic amnesia resulting from unrecognized or undertreated Wernicke encephalopathy and is caused by thiamine (vitamin B1) deficiency. The clinical presentation of thiamine deficiency includes loss of appetite, dizziness, tachycardia, and urinary bladder retention. These symptoms can be attributed to anticholinergic autonomic dysfunction, as well as confusion or delirium, which is part of the classic triad of Wernicke encephalopathy. Severe concomitant infections including sepsis of unknown origin are common during the Wernicke phase. These infections can be prodromal signs of severe thiamine deficiency, as has been shown in select case descriptions which present infections and lactic acidosis. The clinical symptoms of Wernicke delirium commonly arise within a few days before or during hospitalization and may occur as part of a refeeding syndrome. Wernicke encephalopathy is mostly related to alcohol addiction, but can also occur in other conditions, such as bariatric surgery, hyperemesis gravidarum, and anorexia nervosa. Alcohol related Wernicke encephalopathy may be identified by the presence of a delirium in malnourished alcoholic patients who have trouble walking. The onset of non-alcohol-related Wernicke encephalopathy is often characterized by vomiting, weight loss, and symptoms such as visual complaints due to optic neuropathy in thiamine deficiency. Regarding thiamine therapy, patients with hypomagnesemia may fail to respond to thiamine. This may especially be the case in the context of alcohol withdrawal or in adverse side effects of proton pump inhibitors combined with diuretics. Clinician awareness of the clinical significance of Wernicke delirium, urinary bladder retention, comorbid infections, refeeding syndrome, and hypomagnesemia may contribute to the recognition and treatment of the Wernicke-Korsakoff syndrome. Full article

(This article belongs to the Special Issue Wernicke's Encephalopathy and Korsakoff's Syndrome)

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